Gliederung

Objective: Spinal angioblastomas may be encountered as solitary lesions or with multiple lesions in association with von-Hippel-Lindau syndrome (VHL). In the spinal canal, the majority are located inside the spinal cord.

Methods: Hospital files, neuroradiological examinations, intraoperative documentations as well as outpatient examinations were analyzed. The clinical course was documented for each individual symptom and rates for local recurrences were calculated.

Results: Between 1980 and 2010, 1295 spinal tumors were entered into a spinal cord data base. Among these, 27 patients with 32 spinal angioblastomas were encountered (27 intramedullary and 5 extramedullary). Ten of those patients suffered from VHL. Mean age was 41 Â± 13 years and presentation was after an average history of 37 Â± 39 months. Four patients with intramedullary lesions refused an operation. The clinical history is characterized by slowly progressing neurological deterioration often accompanied by pain. All but two operations resulted in a complete resection. Angioblastomas are highly vascularized lesions that should be removed en bloc rather than by piecemeal resection. With large angioblastomas or anterior position, preoperative angiography and embolization should be performed. One patient demonstrated sustained neurological worsening after surgery, five patients improved and the remainder were in stable condition. Mean follow-up was 43 Â± 56 months (maximum 18 years). Postoperative recurrences were observed after 9 operations in 6 patients despite what was considered a complete resection. Four of these patients had VHL.

Conclusions: Spinal angioblastomas are generally associated with a good prognosis after resection. Therefore, an early operation is recommended. Each patient with an angioblastima should be examined for signs of VHL with ophthalmic and abdominal examinations in addition to an MRI of the entire spinal canal and posterior fossa. In VHL patients, the recurrence rate is higher. However, long-term prognosis is determined by extraspinal manifestations of the disease.